scholarly journals A case report of cochlear implantation in patients with osteopetrosis: surgical approaches and auditory results

Author(s):  
Masoud Motasaddi Zarandy ◽  
Hakima Abdullah ◽  
Mina Motasaddi Zarandy ◽  
Mohammadreza Firouzifar ◽  
Farzad Moubedshahi

Background: Osteopetrosis (OP) is a rare disease of the skeletal system that can be associated with complications such as bone fracture, nerve dysfunction and deafness due to increased bone density and reduced bone quality. In this regard and due to the challenge that it can cause for cochlear implantation (CI), in this study we aimed to report CI conducted on two patients with OP in Iran. The Case: Patients were two women diagnosed with OP and bilateral sensorineural hearing loss (SNHL) who underwent CI in the right ear. Preoperative PTA showed a hearing threshold decrease of more than 100 dB in both patients. A standard cochleostomy was performed in one patient and endoscopic surgery in the other patient through the external ear canal. One month after surgery, the hearing threshold improved b y 60−90 dB in both patients. No facial nerve palsy or implant extrusion/migration was observed after surgery. Conclusion: Although technically challenging, CI seems to be a safe and effective method to improve the SNHL in patients with OP. The path for electrode insertion should be tailored to meet the conditions and anatomy of patients. Keywords: Cochlear implantation; osteopetrosis; sensorineural hearing loss

2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.


2021 ◽  
Vol 20 (5) ◽  
pp. 8-12
Author(s):  
T. Yu. Vladimirova ◽  
◽  
A. B. Martynova ◽  

The significance of asymmetric sensorineural hearing loss (ASNHL) is due to a special approach to diagnosis, followed by the process of hearing aids and auditory rehabilitation. Currently, there is no standard audiometric criterion for determining the forms of asymmetry, which significantly affects the assessment of the prevalence of ASNHL. The study aimed to assess the prevalence and classification of ASNHL forms in the older age group using two methods of calculation: 1) the difference in the average hearing threshold at speech frequencies (in the range of 0,5–4 kHz) ≥15 dB was detected in 14,14% of cases; 2) the different degree of hearing loss, according to the International classification, in the right and left ear was 35,98%. In most cases, asymmetry was manifested by bilateral sensorineural hearing loss of varying severity, prevailing in the group of long-livers – 82,6%. Given the potentially high prevalence of asymmetry depending on the audiological criterion, the results of the work are a reason for further research in the development of a unified method for verifying a clinically significant form of ASNHL.


2021 ◽  
Vol 104 (2) ◽  
pp. 260-263

Background: Bacterial meningitis is one of the major factors in the etiology of acquired sensorineural hearing loss in children and adults. Cochlear implantation in these patients is challenging because of inner ear ossification and fibrosis, and this procedure sometimes achieves poorer outcomes in this scenario than with other causes of sensorineural hearing loss. There has been little research into the factors affecting the outcomes of this procedure. Objective: To evaluate the outcomes of cochlear implantation in patients with postmeningitis profound sensorineural hearing loss and to evaluate the factors that affect the results. Materials and Methods: A retrospective review was conducted of thirty patients who were diagnosed with post meningitis profound hearing loss and underwent cochlear implantation at Rajavithi Hospital between 2001 and 2016. Preoperative language status, duration of deafness, preoperative imaging, and degree of electrode insertion were recorded. Categories of auditory performance-II test (CAP-II) was evaluated in all cases, one year postoperative. Results: Thirty postmeningitis deafness patients underwent cochlear implantation. The median age at diagnosis of meningitis and age at implantation were 41 years (range 1 to 75) and 49.50 years (range 3 to 75), respectively. The median duration of deafness was 12 months (range 4 to 300), and the overall mean CAP-II at one year after surgery was 5.47±2.21. The postlinguistic group had a significantly higher CAP-II score than the prelinguistic one (p=0.006). Electrodes were successfully totally inserted in 19 patients (63.3%) and partially inserted in 11 (36.7%). The average CAP-II score in the group with fully-inserted electrodes was significantly higher than in the group with partially-inserted electrodes (p=0.045). There was no correlation between CAP-II score and age at meningitis diagnosis (p=0.069), age at time of surgery (p=0.105), duration of deafness (p=0.506), or preoperative CT (p=0.228) or MRI abnormality (p=0.078). Conclusion: Cochlear implantation in patients with postmeningitis profound hearing loss had high success rates and favorable outcomes. Preoperative language status and degree of electrode insertion were factors that affected auditory performance results. Keywords: Cochlear implantation, Postmeningitis hearing loss, Sensorineural hearing loss, Meningitis, Rajavithi Hospital


2008 ◽  
Vol 19 (06) ◽  
pp. 461-464 ◽  
Author(s):  
Cynthia G. Fowler ◽  
Jennifer L. King

Background: Hearing loss is an infrequently-reported consequence of recreational drug abuse. Although there are sporadic reports of hearing loss from heroin and cocaine ingested separately, there are no reports of hearing loss resulting from the combination of both drugs ingested simultaneously in the form of speedballing. Purpose: The purpose of this report is to document a case of bilateral sensorineural hearing loss associated with an episode of speedballing. Research Design: Case Report Data Collection And Analysis: The subject of this report was a 40-year-old man with a 20-year history of substance abuse. Data collected included a case history, pure tone audiometry, tympanometry and acoustic reflexes, and transient evoked otoacoustic emissions. Results: The audiologic evaluation indicated a mild to moderate, relatively flat, bilateral sensorineural hearing loss that was worse in the right ear. Conclusions: A bilateral sensorineural hearing loss involving both cochlear and neural pathology may be a rare complication of cocaine, heroin, or the combination of the two drugs.


2019 ◽  
Vol 73 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Witold Szyfter ◽  
Michał Karlik ◽  
Alicja Sekula ◽  
Simon Harris ◽  
Wojciech Gawęcki

Introduction: Surgical treatment of deafness by cochlear implants is used for more than 40 years, and during this period permanently, gradual and significant expansion of indications for this surgery has been observed. Material and methods: In our Department in the years 1994-2018 1480 cochlear implantations were performed, both in adults (647) and in children (883). In this study current indications and the rules for eligibility of patients based on 25 years of experience are presented. Results: Indications for cochlear implantation in adults are: 1) bilateral postlingual deafness, 2) bilateral sensorineural hearing loss - in pure tone audiometry > 70 dB HL (average 500-4000 Hz) and in speech audiometry in hearing aids understanding < 50% of words for the intensity of the stimulus 65 dB, in the absence of the benefits of hearing aids, 3) bilateral profound hearing loss for high frequency with good hearing for low frequency, in the absence of the benefits of hearing aids, 4) some cases of asymmetric hearing loss with intensive tinnitus in the deaf ear. An indication in children is bilateral sensorineural hearing loss > 80dB HL confirmed by hearing tests, after about 6 months of rehabilitation with the use of hearing aids. Discussion: Although cochlear implantation is used for more than 40 years, the indications for this treatment underlies constant modifications. They concern the age of eligible patients, implantation in patients with partially preserved hearing, as well as treatment for patients with difficult anatomical conditions. In many countries, bilateral implantations are commonly performed, and more and more centers recommend this treatment in the case of unilateral deafness or asymmetric hearing loss, especially with the accompanying tinnitus in the deaf ear.


1998 ◽  
Vol 77 (4) ◽  
pp. 300-303 ◽  
Author(s):  
Eric W. Sargent ◽  
Douglas L. Beck

Although 1–4% of all cases of sudden sensorineural hearing loss (SSHL) are bilateral, all such patients reported to date have experienced significant recovery of hearing in at least one ear. We report a case of profound, bilateral idiopathic SSHL without recovery which was treated with cochlear implantation; the first such report to our knowledge. The patient achieved open-set spondee recognition. Individuals with sudden bilateral hearing loss in whom treatable causes have been eliminated may benefit from cochlear implantation.


2009 ◽  
Vol 124 (1) ◽  
pp. 105-107 ◽  
Author(s):  
S Cheng ◽  
M da Cruz

AbstractObjective:We report a case of Sweet's disease associated with rapid, profound loss of hearing, against a background of progressive, bilateral, sensorineural hearing loss.Results:The clinical features were indistinguishable from those of immune-mediated inner ear disease. Establishment of a definitive diagnosis was a challenge due to the absence of a reliable diagnostic test. The patient was unresponsive to extensive immunosuppressive therapy and subsequently underwent cochlear implantation, with good hearing outcomes.Conclusions:Profound, bilateral, sensorineural hearing loss in the context of Sweet's disease may be related to the underlying immunological aetiology. Cochlear implantation can successfully restore hearing when immunotherapy fails.


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